Pityriasis rubra pilaris

Pityriasis rubra pilaris (PRP) is a rare skin disorder that causes inflammation and scaling (exfoliation) of the skin.

There are several subtypes of PRP. The cause is unknown, although genetic factors and an abnormal immune response may be involved. One subtype is associated with HIV/AIDS.

Symptoms

PRP is a chronic skin condition in which orange or salmon-colored scaly patches with thick skin develop on the hands and feet.

The scaly areas may cover much of the body. Small islands of normal skin (called islands of sparing) are seen within the areas of the scaly skin. The scaly areas may be itchy. There may be changes in the nails.

PRP can be severe. Although it's not life threatening, PRP can greatly reduce quality of life and limit activities of daily living.

Exams and Tests

The health care provider will examine your skin. Diagnosis is usually made by presence of the unique skin lesions. (A lesion is an abnormal area on the skin). The provider may take samples (biopsies) of the affected skin to confirm the diagnosis and rule out conditions that may look like PRP.

Treatment

Topical creams containing urea, lactic acid, retinoids, and steroids may help. More commonly, treatment includes pills taken by mouth such as isotretinoin, acitretin, or methotrexate. Exposure to ultraviolet light (light therapy) may also help. Drugs that affect the body's immune system are currently being studied and may be effective for PRP.